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 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Calciphylaxis represents a dermatological emergency with a mortality of up to 80%. The disease is characterized by a triad of arteriolar medial calcification, thrombotic cutaneous ischemia and necrotic ulcerations. Recently several mechanisms of vascular calcification have been identified. This may led to preventive measures in the future. Early diagnosis is important to avoid complications such as sepsis. The dermatologist plays an important role in early diagnosis based on the recognition of clinical presentation and histopathology. Patients with end-stage renal disease are most commonly affected by calciphylaxis. The most frequent non-uremic predisposing conditions are primary hyperparathyroidism, malignancies, alcohol-induced liver disease, and autoimmune connective tissue diseases. Medical treatment aims to normalize mineral metabolism to reduce the serum concentration of sodium phosphate and thus to prevent precipitation and calcification. Newer compounds are bisphosphonates, non-sodium/non-aluminium phosphate binders, cinacalcet, paricalcitrol, and sodium thiosulfate. Among the surgical procedures parathyroidectomy did not result in a significant survival benefit. An aggressive surgical debridement of necrotic ulcerations, on the other hand, improved survival. Early diagnosis and a multidisciplinary treatment approach including re-vascularization by the vascular surgeon, repeated surgical debridement and split skin transplantation support wound healing and insure limb conservation.
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PMID:[Cutaneous calciphylaxis]. 2110 56

Calciphylaxis is a disease in which metastatic calcification affects small- and medium-sized vessels resulting in significant dermatologic manifestations. Lesions typically occur over areas of high fat content and progress to black leathery eschars. Calciphylaxis is associated with intense pain and markedly increased risk of infection, often leading to sepsis requiring hospitalization. Diagnosis is made by clinical history and skin biopsy. Management of calciphylaxis is interdisciplinary, emphasizing factors such as primary prevention, proper wound care, pain control, and hormone and mineral balance. Although calciphylaxis carries a high mortality rate, symptomatic treatment has shown promise as a method for controlling disease progression.
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PMID:Calciphylaxis. 2141 Jun 15

Calciphylaxis is a rare disease associated with thrombotic cutaneous ischemia and necrosis. Lesions are usually located on the lower extremities, buttocks and the abdomen. Calciphylaxis is recognized by medial calcification, inflammation and subintimal fibrosis of cutaneous arterioles. Calcification, thrombus formation and occlusion occur sequential before tissue necrosis. The disease is usually observed in patients with end-stage renal disease and hyperparathyroidism. In end-stage renal disease, an elevated parathyroid hormone level, hypercalcemia and hyperphosphatemia direct to vascular mineralization. Calciphylaxis affects about 4% of hemodialysis patients. The clinical syndrome is characterized by a high mortality rate. The most important measure is an active multidisciplinary management approach, with intention to wound care and prevention of sepsis.
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PMID:[Calciphylaxis. Pathogenesis and therapy]. 2168 45

Calciphylaxis is a rare disease primarily affecting patients dependent on dialysis. It is characterised by small vessel media calcification leading to cutaneous ischemia and necrosis. The mortality rate is high with infection and sepsis being the most common causes of death. Calcium salts, vitamin D and high levels of serum calcium and phosphorus increase the risk of calciphylaxis. Current therapies including restoration of mineral homeostasis, wound care and pain control, are not entirely effective. Sodium thiosulfate, by dissolving calcium deposits, is a novel therapeutic choice for calciphylaxis. It has proved successful also in cases refractory to conventional treatment.
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PMID:[Sodium thiosulfate--new hope for the treatment of calciphylaxis]. 2197 87

Calciphylaxis occurs due to calcium deposition in arterioles, which leads to ischemic ulceration of overlying skin. Two-year mortality rates from sepsis ranges from 50% to 80%. Calciphylaxis is most common in hyperparathyroidism secondary to chronic renal impairment and rarely occurs in the setting of normal renal function. Biopsy of the calciphylaxis ulcer reveals calcium deposits lining the vascular intima. Tissue calcification may also be seen on plain radiographs. Calcium-phosphate metabolism should be normalized by treating any underlying hyperparathyroidism with bisphosphonates, parathyroidectomy, and/or cinacalcet in addition to dialysis in chronic renal failure. Intravenous sodium thiosulfate has been used successfully to treat renal and normo-renal calciphylaxis. Sodium thiosulfate displaces calcium ions from calcium deposits to form calcium thiosulfate, which is excreted by the kidneys or dialyzed. Systemic glucocorticoids may prevent ulceration of early plaques of calciphylaxis. Hyperbaric oxygen, skin grafting, and iloprost infusions are useful adjuncts in the management of this debilitating condition.
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PMID:Diagnosis and treatment of calciphylaxis. 2277 99

Calciphylaxis is usually a fatal condition that develops in a few chronic renal failure patients, and it is characterized by calcifications in subcutaneous arteries, infarcts in skin, and the neighboring subcutis. Calciphylaxis, once considered as a rare condition, has been reported to have an annual incidence of 1% and a prevalence of 4% in dialysis patients. We describe our clinical experience in six end-stage renal disease patients on dialysis that presented with calciphylaxis and died due to sepsis, and review the pathogenesis, epidemiology, clinical and histopathologic features, and treatment of calciphylaxis. Physicians should initially consider the possibility of calciphylaxis in case of development of skin lesions in chronic renal failure patients with impaired calcium, phosphorus, and parathyroid hormone levels. The most important cause of mortality in this condition is infection. Therefore, differential diagnosis of these lesions from systemic vasculitis in their early stages and withdrawal of immunosuppressive therapy that increases the tendency to infections are essential.
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PMID:Calciphylaxis: a report of six cases and review of literature. 2315 Nov 46

Calciphylaxis is a devastating disorder with a mortality rate of 80% due to sepsis and organ failure. Hallmarks of this rare disease are arteriolar media calcification, thrombotic cutaneous ischemia, and necrotic ulcerations. Different mechanisms of vascular calcification can lead to calciphylaxis. Early diagnosis by deep cutaneous ulcer biopsy is most important for prognosis. Here, dermatologists play a significant role although treatment usually needs an interdisciplinary approach. Surgical procedures had been the cornerstone of treatment in the past including parathyroidectomy, but recently new medical treatments emerged aiming to normalize disturbances of minerals to reduce the serum concentration of sodium phosphate and to prevent precipitation and calcification. Multimodal therapy is warranted but only aggressive surgical debridement of cutaneous ulcers has shown significant outcome improvement.
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PMID:Update on cutaneous calciphylaxis. 2371 95

Calciphylaxis is an uncommon necrotizing dermal condition, most often related to end-stage renal disease, associated with secondary hyperparathyroidism. The incidence is 1-4% per year in dialyzed patients. The condition is characterized by microcalcification of small and medium-sized blood vessels, causing cutaneous and soft tissue necrosis. The etiology of calciphylaxis is poorly understood, although many reports highlight the abnormal calcium-phosphate products as an important etiological factor. Calciphylaxis is associated with significant morbidity and mortality with estimated 5-year survival rates of less than 50%. Sepsis is the main cause of death. We report two patients of calciphylaxis treated with surgical debridement and excellent outcome in both cases.
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PMID:Photoletter to the editor - Calciphylaxis: a diagnostic and therapeutic challenge. 2385 46

Calciphylaxis is a rare cause of skin ulcerations and necrosis in patients with both normal renal and parathyroid function. Although calciphylaxis appears to be on the increase, treatments are mainly empirical, especially for wound care. The lesions in calciphylaxis are typically very painful and carry a high risk of infection, with sepsis being the leading cause of death in this serious disease. We report two cases of nonuraemic calciphylaxis, which responded to treatment with pamidronate and wound management by negative pressure system.
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PMID:Nonuraemic calciphylaxis: response to treatment with pamidronate and negative pressure therapy. 2547 49

Calciphylaxis (calcific uremic arteriolopathy, CUA) is a rare disease at the interface of nephrology, dermatology and cardiovascular medicine. CUA typically occurs in chronic dialysis patients. However, anecdotal reports also exist about cases in patients without relevant kidney disease. Clinically CUA is characterized by the stepwise development of superficial painful sensations and cutaneous lesions similar to livedo reticularis. Skin necrosis and ulceration represent the full-blown, "late" clinical picture. Panniculitis and circumferential calcification of cutaneous arterioles dominate the histological picture together with endothelial detachment. The prognosis of CUA is poor due to high morbidity and mortality largely resulting from underlying cardiovascular disease or septicemia. The aetiology of CUA is incompletely understood. Previous oral anticoagulation with vitamin K antagonists is considered as a risk factor. Unfortunately, evidence-based therapeutic options are absent, since controlled treatment trials have not been conducted yet. Long-term pain and wound management are mandatory. In the absence of controlled prospective trials registry studies such as the German CUA registry (www.calciphylaxis.net) support data collecting and analysis upon good clinical practice and may stimulate exchange of expertise and networking.
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PMID:[Calciphylaxis]. 2573 78


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